Results 11 to 20 of about 576 (142)

Mechanobullous form of epidermolysis bullosa acquisita: Insights into disease mechanisms as inferred by response to rituximab, but not to JAK inhibitors

JEADV Clinical Practice
Epidermolysis bullosa acquisita (EBA) is a rare autoimmune blistering disease associated with IgG autoantibodies directed against type VII collagen. Different clinical forms have been described, including the classical mechanobullous variant resembling ...
L. Gueissaz, A. Junge, R. Wolf, C. Schlapbach, L. Feldmeyer, L. Borradori   +5 more
doaj   +2 more sources

Hemidesmosome Mutations Contribute to the Onset and Severity of Acquired Autoimmune Bullous Diseases. [PDF]

MedComm (2020)
This study examined hemidesmosome assembly‐related genes in pemphigoid diseases, revealing variants linked to disease onset and severity. Functional analyses, including Caenorhabditis elegans models, Ker‐CT transcriptomics, human proteomics, etc., demonstrated that ITGA6 mutations destabilize hemidesmosomes, disrupt dermal–epidermal adhesion, and ...
Cao S, Wang T, Lv C, Ma S, Yu G, Xia Q, Liu T, Yu Y, Sun L, Pei X, Zhao Q, Wang Z, Wang C, Liu Y, Chen S, Wang J, Zhou G, Liu H, Sun Y, Zhang F.   +19 more
europepmc   +2 more sources

Differential risks of venous thromboembolic events in pemphigus and pemphigoid diseases. [PDF]

J Eur Acad Dermatol Venereol
Journal of the European Academy of Dermatology and Venereology, Volume 40, Issue 4, Page e256-e258, April 2026.
Buenning ME, Purusothaman SH, Symannek S, Curman P, Kridin K, Schmidt E, Bieber K, Ludwig RJ.   +7 more
europepmc   +2 more sources

Bullous Systemic Lupus Erythematosus: Clinical Presentation in Two Asian Cases. [PDF]

Case Rep Med
Bullous systemic lupus erythematosus (BSLE) is a rare subset of systemic lupus erythematosus (SLE), characterized by the rapid onset of vesiculobullous lesions that primarily affect sun‐exposed areas of the body. These lesions typically form along the dermal–epidermal junction and are associated with the presence of autoantibodies that target the skin.
Nguyen PTT, Nguyen TTP, Nguyen HV, Nguyen ATM, Pham NTU.   +4 more
europepmc   +2 more sources

Case Report: Diagnostic and Therapeutic Challenges in Severe Mechanobullous Epidermolysis Bullosa Acquisita

Frontiers in Immunology, 2022
Collagen VII is the main constituent of the anchoring fibrils, important adhesive structures that attach the epidermis to the dermal extracellular matrix.
Franziska Schauer, Alexander Nyström, Manfred Kunz, Stefanie Hübner, Sarah Scholl, Ioannis Athanasiou, Svenja Alter, Judith Fischer, Cristina Has, Dimitra Kiritsi   +9 more
doaj   +1 more source

Generalized erosions, blisters, scarring, and milia in a 65-year-old woman: a diagnostic challenge

Journal of the Egyptian Women’s Dermatologic Society, 2021
Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal blistering disease of the skin and mucous membranes. EBA is caused by antibodies targeting type VII collagen, the major component of anchoring fibrils that connects the basement ...
Galal El Enany, Noha Nagui, Hanan Nada, Marwa M Fawzy, Marwah A Saleh, Iman Sany, Ahmad Nada, Mona Korany, Omar El Ghanam   +8 more
doaj   +1 more source

Topical Application of the PI3Kβ-Selective Small Molecule Inhibitor TGX-221 Is an Effective Treatment Option for Experimental Epidermolysis Bullosa Acquisita

Frontiers in Medicine, 2021
Class I phosphoinositide 3-kinases (PI3K) have been implemented in pathogenesis of experimental epidermolysis bullosa acquisita (EBA), an autoimmune skin disease caused by type VII collagen (COL7) autoantibodies.
Hannah Zillikens, Anika Kasprick, Colin Osterloh, Natalie Gross, Michael Radziewitz, Cindy Hass, Veronika Hartmann, Martina Behnen-Härer, Nancy Ernst, Katharina Boch, Gestur Vidarsson, Remco Visser, Tamás Laskay, Xinhua Yu, Frank Petersen, Ralf J. Ludwig, Katja Bieber   +16 more
doaj   +1 more source

EndoS reduces the pathogenicity of anti-mCOL7 IgG through reduced binding of immune complexes to neutrophils. [PDF]

PLoS ONE, 2014
Endo-β-N-acetylglucosaminidase (EndoS) has been shown to act as a potent pathogen-derived immunomodulatory molecule in autoimmune diseases. Here we investigated how EndoS treatment reduces the pathogenicity of rabbit anti-mCOL7 IgG using different ...
Xinhua Yu, Junfeng Zheng, Mattias Collin, Enno Schmidt, Detlef Zillikens, Frank Petersen   +5 more
doaj   +1 more source

Luteolin peracetate and gossypolone inhibit immune complex-mediated neutrophil activation in vitro and dermal-epidermal separation in an ex vivo model of epidermolysis bullosa acquisita

Frontiers in Immunology, 2023
IntroductionNatural products have been shown to an important source of therapeutics for human disease. In this study, we aimed to identify natural compounds as potential therapeutics for epidermolysis bullosa acquisita (EBA), an autoimmune disease caused
Kai Yang, Junping Yin, Xiaoyang Yue, Katja Bieber, Gabriela Riemekasten, Ralf J. Ludwig, Frank Petersen, Xinhua Yu   +7 more
doaj   +1 more source

Case Report: Biological treatment of epidermolysis bullosa acquisita: report on four cases and literature review

Frontiers in Immunology, 2023
Epidermolysis bullosa acquisita (EBA) is a chronic, recurrent autoimmune subepidermal bullous disease characterized by the presence of autoantibodies targeting type VII collagen -- basement membrane zone antigen.
Konrad Szymański, Cezary Kowalewski, Ewelina Pietrzyk, Katarzyna Woźniak   +3 more
doaj   +1 more source